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Case Report
Gastrointestinal Amyloidosis: An
Unusual Case Where ProteinLosing Enteropathy is Associated to
Malabsorption
Abstract
Introduction: The systemic amyloidosis usually doesnt save the digestive tract, but this
involvement is present in approximately 60% of patients with AA Amyloidosis and only in 8% and 1%
of patients with AL Amyloidosis (respectively through biopsy and clinically). Beside gastrointestinal
involvement is rarely symptomatic and clinical events are not specific.
Enteropathy protein-losing is a rare condition caused by excessive loss of serum protein in
the gastrointestinal tract, resulting in hypoproteinaemia, edema, and, sometimes, pleural/pericardial
effusions. The diagnosis should be taken into account only when other causes have been excluded.
Gastrointestinal AL amyloidosis usually appears with constipation and mechanical obstruction/
pseudobstruction, on the contrary enteropathy protein-losing and malabsorption are rare.
Case report: We report the case of a 59-year -old patient, with AL amyloidosis, who suffered
from weight loss and ascites effusion related to an AL amyloidosis.
Discussion: The case is unusual, both because of the extension in the gastrointestinal tract
and for its unusual clinical presentation as enteropathy protein-losing associated with malabsorption.
Introduction
The systemic amyloidosis usually doesnt save the digestive
tract, but this involvement is present in approximately 60% of
patients with AA Amyloidosis and only in 8% and 1% of patients
with AL Amyloidosis (diagnosis respectively made through biopsy
and clinically) [1]. Beside gastrointestinal involvement is rarely
symptomatic and clinical events are not specific [2].
Enteropathy protein-losing is a rare condition caused by
excessive loss of serum protein in the gastrointestinal tract, resulting
in hypoproteinaemia, edema, and, sometimes, pleural/pericardial
effusions. The diagnosis should be taken into account only when
other causes have been excluded (malnutrition, heavy proteinuria,
and reduced protein synthesis due to liver diseases).
Gastrointestinal AL amyloidosis usually appears with constipation
and mechanical obstruction/pseudobstruction, on the contrary
enteropathy protein-losing and malabsorption are rare [1,2].
Case Presentation
A 59 years old man was admitted for loss weight, taste perversion,
diarrhea. Medical history:
A month earlier, due to an exertional dyspnea, he carried out
an ECG (right ventricular hypertrophy and previous anterior septal
ischemia)
He carried out an echocardiogram, positive for mild cardiac
hypertrophy (apical prevalence), left atrial enlargement, and small
pericardial effusion.
Citation: Daniela T, Vittorio D (2016) Gastrointestinal Amyloidosis: An Unusual Case Where Protein-Losing Enteropathy is Associated to Malabsorption.
Arch Clin Gastroenterol 2(1): 050-052. DOI: 10.17352/2455-2283.000020
050
Discussion
Gastrointestinal tract involvement is almost constant in patients
with systemic amyloidosis (70-100% of patients), but a significant
number is asymptomatic [1-4]. Only 30-60% develop digestive
symptoms [5,6], therefore the diagnosis of digestive amyloidosis is
often difficult. In our case, in fact, it was made quite late.
Symptoms, if present, are aspecific:
Macroglossia (25%)
Our case is characterized by rare clinical manifestation for
AL amyloidosis: a combination of enteropathy protein-losing and
malabsorption.
Enteropathy protein-losing is related to an increased capillary
permeability to plasma proteins, to exudation trough an inflamed
mucosa and, also, to the functional disruption of the lymphatic flow
in the bowel and mesenterium [7].
Gastrointestinal amyloidosis may be localized or widespread [4].
The most common sites of amyloid deposits are the veins and arteries
of the submucosa and muscularis propria. Also lymphatic vessels of
submucosal and subserosal are involved, as well as the muscularis
mucosae and, finally, mucosal or neuromuscular infiltration or
extrinsic autonomic neuropathy [1].
In AL amyloidosis polypoid protrusion, the thickening of the
valvulae conniventes are more common
In AA amyloidosis erosions, mucosal friability and granular
appearance are prevalent
In our case the macroscopic aspect is not usual and is more typical
for AA amyloidosis.
051
Acknowledgements
To Giovanna Fabbretti, Anatomia Patologica of Rimini Hospital
(AUSL Romagna).
Citation: Daniela T, Vittorio D (2016) Gastrointestinal Amyloidosis: An Unusual Case Where Protein-Losing Enteropathy is Associated to Malabsorption.
Arch Clin Gastroenterol 2(1): 050-052. DOI: 10.17352/2455-2283.000020
References
1. Camilleri M, Friedman LS, Grover S (2015) Gastrointestinal amyloidosis:
Clinical manifestations, diagnosis and management.
2. Pavic M, Camus C, Pasquet F, Karkowski L, Galoo E, et al. (2011)
[Seriousness of AL amyloidosis of the digestive system]. Rev Med Interne 32:
e52- e54.
3. Milovic V, Grand RJ, Lamont JT (2013) Protein-losing gastroenteropathy. Up
To Date.
4. Bchade D, Carmoi T, Algayres JP (2008) Amylose digestive (foie exclu).
EMC (Elsevier Masson SAS, Paris), Gastroentrologie 9-089-C-15.
5. James DG, Zuckerman GR, Sayuk GS, Wang HL, Prakash C (2007) Clinical
recognition of AL type amyloidosis of the luminal gastrointestinal tract. Clin
Gastroenterol Hepatol 5: 582588.
6. Repiso A, Valle J, Rodriguez-Merlo R, Munoz-Rosas C, Bolano F, et al.
(2003) Gastrointestinal bleeding and small bowel amyloidosis. Rev Esp
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7. Kawaguchi M1, Koizumi F, Shimao M, Hirose S (1993) Protein-losing
enteropathy due to secondary amyloidosis of the gastrointestinal tract. Acta
Pathol Jpn 43: 333-339.
Copyright: 2016 Daniela T, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and r eproduction in any medium, provided the original author and source are credited.
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Citation: Daniela T, Vittorio D (2016) Gastrointestinal Amyloidosis: An Unusual Case Where Protein-Losing Enteropathy is Associated to Malabsorption.
Arch Clin Gastroenterol 2(1): 050-052. DOI: 10.17352/2455-2283.000020